摘要:

We have previously generated a monoclonal anti-idiotypic antibody (antild 3-17) specific for a cross-reactive idiotype (CRI) present in sera and bound at the basement membrane zone (BMZ) in some patients with bullous pemphigoid (BP). The purpose of the present study was to determine if CRI isolated from sera of patients with BP expressed anti-BMZ activity. CRI, isolated by affinity chromatography from 5 nonrelated patients with BP, bound to the epidermal side of normal human split skin as detected by indirect immunofluorescence. In patients with BP, the presence of the CRI on circulating IgG is associated with anti-BMZ activity

ISSN:1018-8665    

DOI:10.1159/000246935

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We report a case of malignant melanoma that appeared in a 56-year-old man with mycosis fungoides (stage la) during treatment with PUVA. The cumulative UVA dose was 1 177 J/cm2. The pigmented lesion was removed and PUVA therapy discontinued. Histological examination revealed a superficial spreading malignant melanoma (1.77 mm thick, Clark level IV). The delayed-type cutaneous hypersensitivity was studied. The presence of a second malignancy after mycosis fungoides and PUVA therapy may have been coincidental. Nevertheless, this case suggests that the immunosuppression induced by mycosis fungoides and by PUVA therapy might be a pathogenetic factor in the development of malignant melanoma.

ISSN:1018-8665    

DOI:10.1159/000246790

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The role of bullous pemphigoid (BP) autoantibodies (Abs) in the pathogenesis of BP is unclear. Lack of a confirmed experimental animal model prevents studies of the pathogenetic role of BP Abs. We hypothesized that some alterations of BP antigens (BP Ags) will be necessary for causative binding of BP Abs to the Ags. To cause an artificial and reproducible alteration, neonatal mice were irradiated with ultraviolet B (UVB). After the irradiation of 600 mJ/cm2 of UVB, BP serum was intraperitoneally injected. When the BP sera, which recognized only the 230-kD BP Ag, or both 230- and 180-kD BP Ags, were transferred into the UVB-treated mice, erosions and vesicles appeared in 14–78% of the animals. Histopathological examination revealed subepidermal blister formation in the mice treated with UVB and BP sera. Electron microscopy demonstrated that the separation occurred at the level of the lamina lucida. Human IgG and C3 were deposited at the dermal-epidermal junction. Control animals to which healthy sera were injected after the same dose of UVB irradiation showed degeneration of the upper epidermis but no apparent dermal-epidermal cleft formation histopathologically. These results suggest that BP antibodies play a pathogenetic role in vivo. This animal model can contribute to a study of the pathogenesis of B

ISSN:1018-8665    

DOI:10.1159/000246936

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Linear and giant porokeratosis are both rare variants of this disorder of keratinization. We present a case exhibiting the clinical features of both variants coexisting in one patient, which to our knowledge has not been described previously. Furthermore, Bowen’s disease was found to be present in the giant lesion, reflecting the enhanced risk of malignant transformation of this rare subtype of porokeratosi

ISSN:1018-8665    

DOI:10.1159/000246791

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Elastosis perforans serpiginosa (EPS) and the elastotic changes of pseudoxanthoma elasticum (PXE) are rare but well recognized side effects of long-term penicillamine therapy. A 42-year old female patient who developed both of these cutaneous side effects following treatment with high-dose peniciUamine for Wilson’s disease is described; near-complete resolution of the EPS, but not the PXE was achieved by treatment with isotretinoin (0.5 mg/kg/day) for 6 weeks, despite continuation of the penicillamin

ISSN:1018-8665    

DOI:10.1159/000246792

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

To evaluate the significance of the association of malignancy with autoimmune blistering diseases, we studied the incidence of internal malignancies in 496 pemphigus and 1,113 bullous pemphigoid (BP) patients in Japan. Statistical analysis showed that an association ratio of internal malignancies with pemphigus was 5.0% and that with BP was 5.8%. These ratios were significantly higher than that of the controls aged over 70 years (0.61%). Our results indicate that detailed examination for internal malignancy is essential for patients with pemphigus or BP.

ISSN:1018-8665    

DOI:10.1159/000246937

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

We report an unusual benign neoplasm that developed in a child. Tumoral cells were filled with vimentin intermediate filaments. No other features of differentiation were found by immunohistochemistry and electron microscopy. ‘Vimentinoma’ appears to be the most appropriate name for this neopl

ISSN:1018-8665    

DOI:10.1159/000246793

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Pemphigus vulgaris is an autoimmune blistering disease that is characterized by the presence of an antibody against an epidermal cell protein. In our previous studies, we have demonstrated that the presence of the antibody in the patients and in the healthy relatives of the patients strongly correlates with MHC haplotypes. The purpose of this study was to determine the subclasses of IgG present in the sera of patients with active disease, those in remission, healthy and unaffected relatives and normal controls. The presence of the autoantibody to the pemphigus antigen was assayed by a modified, sensitive immunoblot technique. The assay was considered positive if a 130-kD band was seen when normal human epidermis was used as a substrate. In patients with active disease, the sera contained antibodies of the IgG1 and IgG4 subclasses. The sera of patients in remission, those of healthy unaffected relatives and normal controls contained only the IgG1 subclass. The normal controls consist of MHC-matched and non-matched individuals. This study indicates that patients with disease activity have an antibody which is of the IgG1 and IgG4 subclasses, which we consider to be a pathogenic antibody. The sera of healthy relatives and normal controls that contain an antibody which binds to the pemphigus antigen is of the IgG1 subclass only and is considered to be a nonpathogenic or natural autoantibody. These observations provide the basis to study the immunoregulatory mechanisms and the production of normal and pathogenic antibodies.

ISSN:1018-8665    

DOI:10.1159/000246938

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Only 2 cases of histiocytoid hemangioma localized in the nail apparatus have been reported in the literature. We describe here a patient affected by multiple histiocytoid hemangiomas involving the 3rd right digit and fingernail. The pathological features were typical for a diagnosis of histiocytoid hemangioma showing vascular proliferation characterized by enlarged endothelial cells associated with inflammatory cells. Phenotypic characterization of the proliferating cells confirmed their vascular origin.

ISSN:1018-8665    

DOI:10.1159/000246794

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The interactions between lymphocytes and the epidermis are very important in autoimmune skin diseases. Mutual interaction between keratinocytes and T cells is effected both by soluble peptides and by direct cell-to-cell contact. We investigated the possibility that direct cell-to-cell contact with T cells may also play a role in the regulation of keratin gene expression. We have transfected human epidermal keratinocytes with the constructs containing promoters of keratin genes and then cocultured them with the HUT78 strain of human T cells. We found that T cells induce transcription of K5, K6, K14 and K16 genes, as well as the RSV viral promoter, but not K17, K10 or the SV40 viral promoter controls.

ISSN:1018-8665    

DOI:10.1159/000246939

出版商:S. Karger AG    

年代:1994

数据来源: Karger